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CASE REPORT
Year : 2016  |  Volume : 9  |  Issue : 1  |  Page : 114-116  

Cruveilhier - Baumgarten syndrome with multiple splenic artery aneurysms: A case report


Department of Radiodiagnosis, Fortis Hospital, Noida, Uttar Pradesh, India

Date of Web Publication22-Dec-2015

Correspondence Address:
Deepti Madhu Cecil
Department of Radiodiagnosis, Fortis Hospital, Noida, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0975-2870.167983

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  Abstract 

Cruveilhier - Baumgarten (CB) syndrome is a rare medical condition in which liver cirrhosis is associated with portal hypertension causing dilatation of the umbilical and the paraumbilical veins (giant paraumbilical vein). It is important to know about the dilatation of the portosystemic circulation to prevent inadvertent bleeding during any surgical procedure. There are only a few reported cases of CB syndrome and only one with associated multiple splenic artery aneurysms.

Keywords: Giant paraumbilical vein, liver cirrhosis, multiple splenic artery aneurysms, portal hypertension


How to cite this article:
Cecil DM, Chaturvedi A, Kapoor D, Shekhar A. Cruveilhier - Baumgarten syndrome with multiple splenic artery aneurysms: A case report . Med J DY Patil Univ 2016;9:114-6

How to cite this URL:
Cecil DM, Chaturvedi A, Kapoor D, Shekhar A. Cruveilhier - Baumgarten syndrome with multiple splenic artery aneurysms: A case report . Med J DY Patil Univ [serial online] 2016 [cited 2017 Nov 23];9:114-6. Available from: http://www.mjdrdypu.org/text.asp?2016/9/1/114/167983


  Introduction Top


Cruveilhier - Baumgarten (CB) syndrome is a rare clinical disorder characterized by portal hypertension and liver cirrhosis causing dilatation of the umbilical and paraumbilical veins. The term CB disease is reserved for the distention of the paraumbilical veins due to the failure of umbilical vein closure without any evidence of liver disease. Multidetector computed tomography (CT) is a noninvasive method to efficiently assess the overall status of the portosystemic collaterals.


  Case Report Top


A 24-year-old married girl presented to the emergency department with a history of acute onset of lower abdominal pain after meal. There was no history of nausea/vomiting, fever, hematemesis, malena, or jaundice. On examination, her vitals were stable (pulse rate 88/min, respiratory rate 24/min, blood pressure 120/80 mmHg), chest was clear, abdomen was soft with mild periumbilical tenderness, moderate enlargement of liver, and moderate to severe splenomegaly. Ectopic pregnancy was ruled out, in view of the acute lower abdominal pain and her married status, by doing urine pregnancy test. Abdominal and pelvic ultrasonography were also performed which showed enlarged liver with coarse echotexture, massive splenomegaly, dilated portal vein, and enlarged collaterals. There was no ascites and pelvic organs were normal.

Laboratory investigations done were as follows: Hemoglobin 12.5 g/dL, total leukocyte count 5500/µL, platelet count 50,000/µL, international normalized ratio 1.2, total bilirubin 0.76 mg/dL, direct bilirubin 0.14 mg/dL, aspartate aminotransferase 41, total serum protein 6.0 g/dL, and serum albumin 3.4 g/dL.

CT scan was performed on the following day on multidetector 64 slice CT scanner. It showed chronic liver disease with dilated portal vein (26 mm diameter), dilated splenic vein (23 mm diameter), gross splenomegaly with craniocaudal span of 22 cm, re-canalization of paraumbilical vein (giant paraumbilical vein) with infraumbilical collateral venous channels draining into bilateral external iliac veins and dilated and tortuous splenic artery with multiple small intra- and extra-splenic aneurysms.


  Discussion Top


Cruveilhier - Baumgarten syndrome is an uncommon condition with very few cases reported in literature. It was first described by Pégot in 1833, and then by Jean Cruveilhier (1835) and Paul Clemens von Baumgarten (1907). However, the first complete review in literature was done by Armstrong et al. in 1942, who is credited with coining of the term CB syndrome and CB disease. [1]

CB syndrome is a rare complication of liver cirrhosis. [2] It is characterized by the presence of liver disease or portal hypertension leading to spontaneous portosystemic collateralization between paraumbilical vein, periumbilical veins of the anterior abdominal wall and the epigastric veins draining into the external iliac veins [Figure 1] and [Figure 2]. In a classic CB syndrome, the umbilical portion of the left portal vein feeds the paraumbilical vein that then leaves the liver and extends toward the umbilicus [Figure 3]. [3] The development of large recanalized paraumbilical vein has been found to prevent formation of bleeding esophageal varices and also prevent predisposition to hepatic encephalopathy. [4] It is therefore considered an acceptable means of decompression of the portal venous system without associated gastrointestinal bleeding. [5]
Figure 1: (a) Coronal and (b) axial contrast-enhanced computed tomography images show the giant recanalized paraumbilical vein communicating with the external iliac veins through epigastric veins

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Figure 2: Three-dimensional volume rendering images showing the giant recanalized paraumbilical vein (a) and splenomegaly (b)

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Figure 3: Coronal contrast-enhanced computed tomography images show dilated left portal vein feeding the giant recanalized paraumbilical vein. Splenomegaly and tortuous splenic artery also noted

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The clinical importance lies in the fact that these subcutaneous collaterals may undergo spontaneous hemorrhage or inadvertent significant bleeding during abdominal surgery or paracentesis. [6] Since portosystemic varices develop by means of distention and elongation of preexisting small veins, the variceal walls are thin. These vessels are easily torn and difficult to repair. There have been many reported cases of intraoperative mortality due to accidental disruption of an unexpected varix. [7]

The patients usually present with nonspecific history like feeling a lump in the left side of abdomen. [3]

However, in our case, the patient was asymptomatic and presented to the emergency department with unrelated complaints. Apart from the classic finding of giant paraumbilical vein joining, the systemic circulation through the external iliac veins via bilateral inferior epigastric veins, the CT scan showed uncommon finding of multiple small, intra- and extra-parenchymal splenic artery aneurysms (SAAs) [Figure 4]. Only a few cases of multiple SAAs associated with portal hypertension have been reported in literature. The prevalence of SAAs ranges from 0.2% to 10.4%. The exact causative factor for SAAs is not known, but they are usually associated with hypertension, portal hypertension, cirrhosis, liver transplantation, and pregnancy. [8] Rupture of the aneurysm is a major complication and when associated with portal hypertension, the mortality rate can be as high as 56%. Management is conservative, unless:
Figure 4: Coronal maximum intensity projection (a) and threedimensional volume rendering image (b) showing multiple small splenic aneurysms

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  1. They become symptomatic,
  2. Are larger than 2 cm in size,
  3. Are diagnosed during pregnancy, or
  4. Show increase in dimension during follow-up.
The treatment options are conventional open surgery, endovascular therapy, or a combination of both. [9]

 
  References Top

1.
Armstrong EL, Adams WL, Tragerman LJ, Townsend EW. The Cruveilhier-Baumgarten syndrome. Ann Intern Med 1942;1:113-51.  Back to cited text no. 1
    
2.
Wilairatana P, Tangpukdee N, Jittaseree L. Cruveilhier-Baumgarten syndrome in a cirrhotic patient taking alcohol with Boesenbergia pundurata. Southeast Asian J Trop Med Public Health 2010;41:1267-70.  Back to cited text no. 2
    
3.
Singla V, Galwa RP, Saxena AK, Khandelwal N. Cruveilhier Baumgarten syndrome with giant paraumbilical vein. J Postgrad Med 2008;54:328-9.  Back to cited text no. 3
[PUBMED]  Medknow Journal  
4.
Lam KC, Juttner HU, Reynolds TB. Spontaneous portosystemic shunt: Relationship to spontaneous encephalopathy and gastrointestinal hemorrhage. Dig Dis Sci 1981;26:346-52.  Back to cited text no. 4
[PUBMED]    
5.
Cho KC, Patel YD, Wachsberg RH, Seeff J. Varices in portal hypertension: Evaluation with CT. Radiographics 1995;15: 609-22.  Back to cited text no. 5
    
6.
Sodhi JS, Zarger SA, Khan MA, Javid G, Khan BA, Shah AH, et al. Cruveilheir-Baumgarten syndrome revisited. Indian J Gastroenterol 2007;26:173.  Back to cited text no. 6
    
7.
Henseler KP, Pozniak MA, Lee FT Jr, Winter TC 3 rd . Three-dimensional CT angiography of spontaneous portosystemic shunts. Radiographics 2001;21:691-704.  Back to cited text no. 7
    
8.
Agrawal GA, Johnson PT, Fishman EK. Splenic artery aneurysms and pseudoaneurysms: Clinical distinctions and CT appearances. AJR Am J Roentgenol 2007;188:992-9.  Back to cited text no. 8
    
9.
Yakubovitch D, Halak M, Khaikin M, Silverberg D. Multiple splenic artery aneurysms in a patient with portal hypertension. Isr Med Assoc J 2013;15:55-6.  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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Case Report
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